Recombinant humaan Aminoacylase/ACY1-eiwit

Aminoacylase-1 is a cytosolic, homodimeric, zinc-binding enzyme that catalyzes the hydrolysis of acylated L-amino acids to L-amino acids and acyl group, and has been postulated to function in the catabolism and salvage of acylated amino acids. Defects in ACY1 are the cause of aminoacylase-1 deficiency (ACY1D) . ACY1D results in a metabolic disorder manifesting with encephalopathy, unspecific psychomotor delay, psychomotor delay with atrophy of the vermis and syringomyelia, marked muscular hypotonia or normal clinical features. Epileptic seizures are a frequent feature. All affected individuals exhibit markedly increased urinary excretion of several N-acetylated amino acids. Recombinant human ACY1 protein, fused to His-tag at N-terminus, was expressed in E. coli and purified by using conventional chromatography techniques.

Aminoacylase 1, ACY1D, ACYLASE, Aminoacylase 1

E.coli

Human

His-Tag

SDS-PAGE

0.5 mg/mL (determined by Bradford assay)

> 90% by SDS-PAGE

48kDa (428aa) confirmed by MALDI-TOF

Aminoacylase, ACY1, Aminoacylase 1, ACY1D, ACYLASE, Aminoacylase 1, ATGP0659-10 µg, ATGP0659-20 µg, ATGP0659-50 µg, ATGP0659-100 µg, ATGP0659-250 µg, ATGP0659-500 µg, ATGP0659-1 mg, ATGP0659-10, ATGP0659-20, ATGP0659-50, ATGP0659-100, ATGP0659-250, ATGP0659-500, ATGP0659-1

Sass J.O., et al. (2006) Am. J. Hum. Genet. 78:401-409; ; Van Coster RN., et al. (2005) Biochem Biophys Res Commun. 338 (3) :1322-6;

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Liquid in. Phosphate-Buffered Saline (pH 7.4) containing 10% glycerol

Enzymes & Proteases

NP_000657.1

Q03154

Human

TSKGPEEEH PSVTLFRQYL RIRTVQPKPD YGAAVAFFEE TARQLGLGCQ KVEVAPGYVV TVLTWPGTNP TLSSILLNSH TDVVPVFKEH WSHDPFEAFK DSEGYIYARG AQDMKCVSIQ YLEAVRRLKV EGHRFPRTIH MTFVPDEEVG GHQGMELFVQ RPEFHALRAG FALDEGIANP TDAFTVFYSE RSPWWVRVTS TGRPGHASRF MEDTAAEKLH KVVNSILAFR EKEWQRLQSN PHLKEGSVTS VNLTKLEGGV AYNVIPATMS ASFDFRVAPD VDFKAFEEQL QSWCQAAGEG VTLEFAQKWM HPQVTPTDDS NPWWAAFSRV CKDMNLTLEP EIMPAATDNR YIRAVGVPAL GFSPMNRTPV LLHDHDERLH EAVFLRGVDI YTRLLPALAS VPALPSDS